What is sickle cell disease?
Sickle cell disease is a genetic red blood cell disorder. It changes normal, round red blood cells into cells shaped like crescent moons. The name "sickle cell" comes from the crescent shape of the cells. (A sickle is a tool with a crescent-shaped blade.)
Normal red blood cells move easily through your blood vessels, taking oxygen to every part of your body. But sickled cells can get stuck and block blood vessels, which stops the oxygen from getting through. That can cause a lot of pain. It can also harm organs, muscles, and bones.
Sickle cell disease can cause other health problems, such as infections, anemia, and stroke.
What are the symptoms of sickle cell disease?
When a child is born with sickle cell disease, no one can predict which symptoms will appear, when they will start, or how bad they will be.
Painful events (sickle cell crises) in the hands or feet, belly, back, or chest are the most common symptom of sickle cell disease. They are periods of pain that happen when sickled cells get stuck in blood vessels and block the blood flow. This pain may last from hours to days.
Most people with sickle cell disease are anemic. Anemia can make you feel weak and tired. People with sickle cell anemia may look pale or washed out. They may be short of breath. Their skin and the whites of their eyes may have a yellowish look (jaundice).
How is sickle cell disease diagnosed?
Sickle cell disease is diagnosed when initial blood tests show abnormal hemoglobin. A sickle cell test looks for sickle cell trait and sickle cell disease.
The disease can be diagnosed soon after birth. A sample of blood is taken from the infant's heel. It's sent to a lab. There it is screened for the presence of sickle cell hemoglobin (hemoglobin S).
The disease may also be diagnosed based on the problems it can cause. More testing may be done if needed.
Doctors can diagnose sickle cell disease before a child is born (prenatally). Prenatal tests include amniocentesis and chorionic villus sampling (CVS).
Couples who are at risk for passing on this disease to their children may want to talk with a genetic counselor about prenatal testing before getting pregnant.
How is sickle cell disease treated?
Medical treatment can help you manage pain and avoid other health problems from sickle cell disease.
Early treatment includes daily antibiotics from 2 months to 5 years of age to help prevent infections. Routine childhood and adult immunizations are also important. Your doctor may also suggest you take folic acid supplements.
Medicines that treat sickle cell disease include hydroxyurea and pain medicines. You may need a prescription for some of the medicines. Others you can buy over-the-counter. Pain medicine may work best when you also do other things to manage pain. You can prepare for painful events ahead of time by creating a pain management plan with your doctor.
Some people need regular blood transfusions to lower the risk of stroke and to treat anemia and other problems. Some people take prescription medicine, such as L-glutamine (Endari), to prevent problems. In rare cases, a stem cell transplant might be an option.
How can you care for yourself when you have sickle cell disease?
- Take your medicines exactly as prescribed. Call your doctor if you think you are having a problem with your medicine.
- Take pain medicines exactly as directed.
- If the doctor gave you a prescription medicine for pain, take it as prescribed.
- If you are not taking a prescription pain medicine, ask your doctor if you can take an over-the-counter medicine.
- Try to help ease pain by distracting yourself. Use guided imagery, deep breathing, and relaxation exercises. A pain specialist can teach you pain management skills.
- Avoid alcohol. It can make you dehydrated.
- Dress warmly in cold weather. The cold and windy weather can lead to severe pain.
- Do not smoke. Smoking can reduce the amount of oxygen in your blood. If you need help quitting, talk to your doctor about stop-smoking programs and medicines. These can increase your chances of quitting for good.
- Get plenty of sleep.
- Get regular eye exams. Sickle cell disease can cause vision problems.
- Wear medical alert jewelry that says that you have sickle cell disease. You can buy this at most drugstores.
- Avoid infections such as COVID-19, colds, and the flu. Wash your hands often. Get a pneumococcal vaccine shot. If you have had one before, ask your doctor whether you need another dose. Get a flu shot every year. Stay up to date on your COVID-19 vaccinations.
Sickle cell disease in children: When to call
Call 911 anytime you think your child may need emergency care. For example, call if:
- Your child has symptoms of a severe problem from sickle cell.
- Your child has symptoms of a stroke. These may include:
- Sudden numbness, tingling, weakness, or loss of movement in the face, arm, or leg, especially on only one side of his or her body.
- Sudden vision changes.
- Sudden trouble speaking.
- Sudden confusion or trouble understanding simple statements.
- Sudden problems with walking or balance.
- A sudden, severe headache that is different from past headaches.
- Your child is in severe pain.
- Your child has symptoms of a heart attack. These may include:
- Chest pain or pressure, or a strange feeling in the chest.
- Sweating.
- Shortness of breath.
- Nausea or vomiting.
- Pain, pressure, or a strange feeling in the back, neck, jaw, or upper belly or in one or both shoulders or arms.
- Lightheadedness or sudden weakness.
- A fast or irregular heartbeat.
Call your doctor now or seek immediate medical care if:
- Your child has a fever.
Watch closely for changes in your child's health, and be sure to contact your doctor if your child is having any problems.
©2011-2025 Healthwise, Incorporated